Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rhabdomyosarcoma is the most common malignant orbital tumor of childhood. It has twice been reported to arise within the globe from the iris. In addition, teratoid medulloepithelioma, a tumor arising from the ciliary epithelium, can contain a rhabdomyoblastic component, often in combination with other heteroplastic elements. The authors report what may be the first recorded case of an embryonal rhabdomyosarcoma of the ciliary body, possibly representing a one-sided differentiation of a malignant teratoid medulloepithelioma.
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PMID:Rhabdomyosarcoma of the ciliary body. 225 20

The present paper reports a case of rhabdomyosarcoma in a 4-year-old patient, the rapidity with which it spread and the intensity of its extension bringing the case under observation. It is, in fact, a case of embryonal rhabdomyosarcoma localized in the right parotid area with an extension to the temporal fossa, to the epi-oro-hypopharynx as far as the lateral vertebral region with C5-C6 involvement and penetrating the medium cranialis fossa. Initially the child showed signs of tissue growth at the right parotid site, originating without symptoms and rapidly increasing in volume. As exploratory surgery revealed that it would be impossible to achieve radical removal of the growth, the authors opted for radio-chemotherapy treatment. This type of approach, however, gives short lived results, in this case evidenced by recurrences within six months and leading to the patient's death after just eight months. It is important to stress the rapid evolution of this neoplasm, its tendency to recur and metastasize as well as its poor prognosis in general.
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PMID:[A case of rhabdomyosarcoma of the parotid region]. 226 Apr 44

The paper describes a case of embryonal rhabdomyosarcoma of the maxillary antrum which, according to the Intergroup Rhabdomyosarcoma Study (IRS) protocol, was treated with a protocol which included cycles of chemotherapy and radiotherapy.
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PMID:[Case of rhabdomyosarcoma of the maxillary bone]. 227 51

Rhabdomyosarcoma of the biliary tree is a rare cause of biliary tract obstruction in childhood. A 3-year-old child is reported here after presenting with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the ampulla of Vater. He underwent pancreaticoduodenectomy followed by adjuvant chemotherapy and irradiation. He is now well and free of disease 5 years following treatment. This child appears to be the first long-term survivor who has required pancreaticoduodenal resection for this lesion.
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PMID:Embryonal rhabdomyosarcoma of the ampulla of Vater with long-term survival following pancreaticoduodenectomy. 228 99

A parent rhabdomyosarcoma cell line designated SCMC-RM2 was established from bone-marrow tumor cells taken from an 11-year-old girl with an embryonal rhabdomyosarcoma. Subsequently a cloned SCMC-RM2-1 cell line was isolated from a parent line. These cell lines grew as adherent monolayers in liquid culture with a doubling time of 50 and 52 hr, respectively. In addition, colonies were established in soft agar, which grew in a dose-dependent fashion with a cloning efficiency of 0.7 and 0.8%, respectively. Chromosomal analysis showed these cell lines had neither double minutes nor homogeneously staining regions. Chromosome number ranged from 61 to 93, translocation; t(9;13)(p22;q14) was identified, and no alteration of chromosome 2 was observed. Surface membrane antigen profile of parent and cloned lines by using a panel of 24 monoclonal antibodies (MAbs) excluded the possibility of these being neuroblastoma cell lines. In addition, MAbs to the cytoplasmic protein desmin, myoglobin, muscle actin (alpha and gamma) and alpha-sarcomeric actin reacted with these cell lines, SCMC-RM2 and SCMC-RM2-1 being thus identified as rhabdomyosarcoma. Southern blot analyses revealed 8- and 7-fold amplification of the N-myc gene in SCMC-RM2 and SCMC-RM2-1 as compared with the promyelocytic cell line HL60. Over-expression of the N-myc mRNA was noted over control cell lines.
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PMID:Characterization of an embryonal rhabdomyosarcoma cell line showing amplification and over-expression of the N-myc oncogene. 232 48

A 15-year-old boy was referred to the ear, nose, and throat clinic because of a swelling in the upper premolar region. The initial diagnosis of a poorly differentiated soft-tissue sarcoma was made. Further immunohistochemical studies established a definitive diagnosis of embryonal rhabdomyosarcoma. The tumor cells coexpressed both desmin, the component of muscle type intermediate filaments, and vimentin, which is typically found in mesenchymal tissues. Such coexpression is found in the early stages of myogenic differentiation. Another cytoskeletal protein, actin, was also found in relatively high concentrations. These results suggested the possible use of antibodies to these cytoskeletal proteins as histogenetic markers for the diagnosis of poorly differentiated rhabdomyosarcoma.
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PMID:Embryonal rhabdomyosarcoma: immunohistochemical characterization. 241 9

A case of prostatic rhabdomyosarcoma in a 5-year-old boy is reported. He was brought to our clinic on Apr. 1, 1982 with complaints of pollakisuria and urethral pain. X-ray examinations revealed a huge intrapelvic tumor, and it was histopathologically diagnosed as embryonal rhabdomyosarcoma with a specimen of transrectal needle biopsy. Since the tumor was too huge to resect completely, he was initially treated with combination chemotherapy regimen of vincristine, actinomycin D and cyclophosphamide (VAC therapy), and resulted in failure. Then another combination chemotherapy consisting of cis-diamminedichloroplatinum, vinblastine and bleomycin (PVB therapy) was tried, and the tumor showed reduction in size. On Oct. 15, 1982, total cystectomy with ileal conduit urinary diversion was performed. Histopathologically, degenerative change and partial necrosis of the tumor cell were recognized. After the operation, he was treated with radiation therapy and prophylactic VAC therapy. But six months later, multiple pulmonary metastases occurred and gradually increased in size and number. They did not respond to any other chemotherapy. He died on July 13, 1983. We discussed the chemotherapy for rhabdomyosarcoma, and emphasized that the PVB therapy should be tried on rhabdomyosarcoma as an initial chemotherapy.
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PMID:[Combination chemotherapy with cis-diamminedichloroplatinum, vinblastine and bleomycin for a rhabdomyosarcoma of the prostate in a child: report of a case]. 241 60

Cultured human embryonal rhabdomyosarcoma cells express the basic fibroblast growth factor (bFGF) gene and they produce bFGF, which is apparently composed of two microheterogenous forms with Mrs of 16,500 and 17,200, respectively. bFGF derived from the rhabdomyosarcoma cells stimulates their own proliferation and that of human or bovine vascular endothelial cells. It is conceivable that the rhabdomyosarcoma-derived bFGF stimulates the growth and neovascularization of human rhabdomyosarcomas and that it may thereby contribute to the development of these tumors.
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PMID:Basic fibroblast growth factor in human rhabdomyosarcoma cells: implications for the proliferation and neovascularization of myoblast-derived tumors. 243 91

A case of prostatic rhabdomyosarcoma in a 5-year-old boy is presented. He was referred to us because of complete urinary retention. Histologically, embryonal rhabdomyosarcoma of the prostate was found. Initially, he was treated with combination chemotherapy consisting of vincristine, actinomycin-D, adriamycin and radiation therapy. This therapy reduced the size of the tumor markedly. Therefore, we performed prostatectomy. No tumor cells were found on the surgical margin of the resected prostate. About 11 months after the operation, rapidly growing recurrent lesions were found near the bladder neck. Bilateral hydronephrosis and an intravesical massive space occupying lesion were found on the excretory pyelograms and cystogram. Then three drug combination chemotherapy consisting of vinblastine, cis-diamminedichloroplatinum and bleomycin (PVB therapy) was administered and the first course of the chemotherapy reduced the size of the recurrent tumor. Drainage of contrast medium from bilateral kidneys became smooth, and no gross hematuria or severe frequency was observed. Total cystectomy with ileal conduit urinary diversion was performed. Four months after this operation, metastatic lesions appeared at the pubic bone, right ischiadic bone and rectum, and they were resected operatively. However, multiple pulmonary metastases soon occurred, and he did on June 30, 1985, approximately 2 years after the first diagnosis. We have discussed the effectiveness of combination chemotherapy, especially PVB therapy for recurrent cases.
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PMID:[Rhabdomyosarcoma of the prostate in a child: report of a case]. 244 85

Five hundred thirteen soft tissue tumours of childhood referred to the International Society of Pediatric Oncology (SIOP) Rhabdomyosarcoma study have been reviewed. The period covered was from January 1975 to December 1983. Three hundred thirty-nine neoplasms were regarded as embryonal rhabdomyosarcoma. The histological diagnosis of all referred tumours is given, and a classification of rhabdomyosarcomas developed during the course of the study is described. The relationship between histological subtypes and aspects of clinical behaviour is presented. The classification is thought to be helpful in diagnosis and also in assessment of the likelihood of recurrence and/or metastasis of childhood rhabdomyosarcoma.
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PMID:Histopathological classification of childhood rhabdomyosarcoma: a report from the International Society of Pediatric Oncology pathology panel. 247 74


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